scholarly journals IgG4-Related Sclerosing Cholangitis Mimicking Cholangiocarcinoma

2021 ◽  
pp. 39-46
Author(s):  
Pham Minh Thong ◽  
Vu Dang Luu ◽  
Thieu-Thi Tra My ◽  
Nguyen Xuan Hien ◽  
Tran Anh Tuan ◽  
...  
Keyword(s):  
Bile Duct ◽  
Sclerosing Cholangitis ◽  
Wall Thickening ◽  
Systemic Involvement ◽  
Bile Duct Stenosis ◽  
Diffusion Weighted Images ◽  
Igg4 Related Disease ◽  
Imaging Characteristics ◽  
Serum Igg4 ◽  
Duct Stenosis

IgG4-related sclerosing cholangitis (IgG4-SC) is a relatively newly identified disease that is frequently associated with autoimmune pancreatitis. The differential diagnosis between cholangiocarcinoma, primary sclerosing cholangitis, and IgG4-SC can be challenging due to significant overlap among the clinical and imaging characteristics. We report the case of a 71-year-old woman who was diagnosed with IgG4-related disease based on increased serum IgG4 levels, imaging, and clinical presentation, which showed systemic involvement, including sclerosing cholangitis and kidneys. The patient presented with chronic jaundice. Magnetic resonance imaging revealed bile duct strictures and the dilatation of upstream bile ducts, smooth wall thickening with uniform enhancement in the delayed phase, and no vascular infiltration. Multiple low-density, wedge-shaped areas were identified in both kidneys, which were hypointense on T2-weighted images and hyperintense on diffusion-weighted images. The serum IgG4 levels of this patient were elevated to nearly 10-fold the normal upper limit. A diagnosis of IgG4-SC associated with IgG4-related kidney was made. Based on this case, pre-surgery IgG4 serum treatment in patients with non-malignant bile duct stenosis was recommended to exclude IgG4-SC.

scholarly journals Difficulty Achieving a Preoperative Diagnosis of IgG4-Related Sclerosing Cholangitis

2018 ◽  
Vol 12 (2) ◽  
pp. 425-431
Author(s):  
Kazuhiro Suzumura ◽  
Etsuro Hatano ◽  
Masaharu Tada ◽  
Hideaki Sueoka ◽  
Hiroshi Nishida ◽  
...  
Keyword(s):  
Bile Duct ◽  
Preoperative Diagnosis ◽  
Sclerosing Cholangitis ◽  
Wall Thickening ◽  
Distal Bile Duct ◽  
Obliterative Phlebitis ◽  
Bile Duct Stenosis ◽  
Storiform Fibrosis ◽  
Lymphoplasmacytic Infiltration ◽  
Duct Stenosis

A 75-year-old male was admitted to our hospital because of bile duct stenosis. He had no medical history of autoimmune disease. The level of tumor markers, serum IgG, and IgG4 were within normal ranges. Computed tomography showed perihilar and distal bile duct stenosis and wall thickening without swelling or abnormal enhancement of the pancreas. Endoscopic retrograde cholangiopancreatography showed perihilar and distal bile duct stenosis. A biopsy and cytology from the distal bile duct stenosis suggested adenocarcinoma, and cytology from the perihilar bile duct also suggested adenocarcinoma. A preoperative diagnosis of perihilar and distal bile duct cancer was made, and the patient underwent left hepatectomy and pancreaticoduodenectomy. Resected specimens showed wall thickening in the perihilar and distal bile duct; however, tumors were unclear. A histopathological examination revealed lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis in the perihilar and distal bile ducts. Immunohistochemistry revealed diffuse infiltration of IgG4-positive plasma cells in the perihilar and distal bile ducts. Lymphoplasmacytic infiltration, inflammatory change, storiform fibrosis, and obliterative phlebitis were shown in the pancreas. A final diagnosis of IgG4-related sclerosing cholangitis (IgG4-SC) with autoimmune pancreatitis was made. We herein report a case in which a preoperative diagnosis of IgG4-SC was difficult due to normal serum IgG4 levels and no obvious pancreatic lesion.

scholarly journals Usefulness of Steroid Administration for Diagnosis of IgG4-Related Sclerosing Cholangitis

2012 ◽  
Vol 97 (2) ◽  
pp. 145-149 ◽  
Author(s):  
Norio Kubo ◽  
Hideki Suzuki ◽  
Tsutomu Kobayashi ◽  
Kenichiro Araki ◽  
Shigeru Sasaki ◽  
...  
Keyword(s):  
Bile Duct ◽  
Steroid Therapy ◽  
Sclerosing Cholangitis ◽  
Liver Volume ◽  
Immunoglobulin G4 ◽  
Steroid Administration ◽  
Bile Duct Stenosis ◽  
Serum Igg4 ◽  
Hilar Bile Duct ◽  
Duct Stenosis

Abstract Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is one of the IgG4-related systemic sclerosing diseases and responds well to steroid therapy. A 58-year-old male was admitted with hilar bile duct stenosis revealed by computed tomography. We performed percutaneous transhepatic right portal vein embolization (PTPE) and scheduled a right hepatectomy because a hilar cholangiocarcinoma was first suspected. However, there was no cytologic evidence of malignancy and serum IgG4 was elevated. Steroid therapy was initiated after PTPE. There was no evidence of bile duct stenosis after 4 weeks. Improving diagnostic technique, IgG4-SC was diagnosed and treated with steroid therapy. In some cases, we couldn't deny the malignancy and performed unnecessary resection. We recommend that steroid administration while waiting for the liver volume to increase after PTPE is useful. The therapy aids in the diagnosis of bile duct stenosis, which has value for a hilar bile duct limit type of IgG4-SC, as in the case reported here.

scholarly journals Intraductal Papillary Neoplasm of the Bile Duct With Invasive Adenocarcinoma Complicating IgG4-related Sclerosing Cholangitis: Report of a Case

2017 ◽  
Vol 102 (1-2) ◽  
pp. 70-76
Author(s):  
Ryo Ashida ◽  
Teiichi Sugiura ◽  
Yukiyasu Okamura ◽  
Takaaki Ito ◽  
Keiko Sasaki ◽  
...  
Keyword(s):  
Bile Duct ◽  
Sclerosing Cholangitis ◽  
Invasive Carcinoma ◽  
Extrahepatic Bile Duct ◽  
Inflammatory Cells ◽  
Wall Thickening ◽  
Multidetector Row Computed Tomography ◽  
Intraductal Papillary Neoplasm ◽  
Serum Igg4 ◽  
Papillary Neoplasm

Although there have been many previous studies of IgG4-related SC focusing on the differential diagnosis from cholangiocarcinoma, only a few patients with cholangiocarcinoma against a background of IgG4-related SC have been reported. We herein present a case of intraductal papillary neoplasm of the bile duct (IPNB) associated with invasive carcinoma complicating IgG4-related sclerosing cholangitis. A 71-year-old female with icterus was admitted to a local hospital, where stricture of the extrahepatic bile duct were detected, and subsequently referred to our hospital for possible surgery. Abdominal multidetector-row computed tomography demonstrated marked wall thickening along the entire extrahepatic bile duct. The left lateral superior bile duct (B2) and left lateral inferior duct (B3) were individually obstructed, and percutaneous transhepatic biliary drainage catheters were placed in B2 and B3 separately. The patient was diagnosed to have diffusely spread cholangiocarcinoma and underwent right hepatic trisectionectomy with caudate lobectomy and pancreatoduodenectomy. A histological examination revealed intraductal papillary tumors composed of fibrovascular stalks covered by neoplastic epithelium. Carcinomatous invasion of the papillary tumors was observed in the fibromuscular layer, and there was abundant infiltration of inflammatory cells with fibrosis outside of the cancerous tissue. The inflammatory cells were primarily composed of plasma cells, a majority of which were positive for IgG4 (>30 cells/high-power field); the postoperative serum IgG4 level was 890 mg/dL. Therefore, a diagnosis of coexisting IPNB associated with invasive carcinoma and IgG4-related sclerosing cholangitis was made. To the best of our knowledge, this is the first report of IPNB complicating IgG4-related sclerosing cholangitis.

scholarly journals The Utility of Serum IgG4 Concentrations as a Biomarker

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Shigeyuki Kawa ◽  
Tetsuya Ito ◽  
Takayuki Watanabe ◽  
Masahiro Maruyama ◽  
Hideaki Hamano ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
High Serum ◽  
Wall Thickening ◽  
Systemic Involvement ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Long Term Follow Up ◽  
Characteristic Features

IgG4-related disease is a new disease entity involving IgG4 in its clinical presentation and having 6 characteristic features: (1) systemic involvement; (2) solitary or multiple lesions showing diffuse or localized swelling, masses, nodules, and/or wall thickening on imaging; (3) high serum IgG4 concentration >135 mg/dL; (4) abundant infiltration of lymphoplasmacytes and IgG4-bearing plasma cells; (5) a positive response to corticosteroid therapy; and (6) complications of other IgG4-related diseases. To date, most IgG4-related diseases have been recognized as extrapancreatic lesions of autoimmune pancreatitis. This paper will discuss the utility of IgG4 as a biomarker of IgG4-related diseases, including in the diagnosis of autoimmune pancreatitis and its differentiation from pancreatic cancer, in the prediction of relapse, in the long-term follow-up of patients with autoimmune pancreatitis and normal or elevated IgG4 concentrations, and in patients with autoimmune pancreatitis and extrapancreatic lesions, as well as the role of IgG4 in the pathogenesis of IgG4-related disease.

scholarly journals Diagnosis of Cancer Spread Using Percutaneous Transhepatic Biliary Cholangioscopy-guided Ultrasonography for Malignant Bile Duct Stenosis

2001 ◽  
Vol 7 (3-4) ◽  
pp. 159-164 ◽  
Author(s):  
Hirokazu Inoue
Keyword(s):  
Bile Duct ◽  
Extrahepatic Bile Duct ◽  
Biliary Duct ◽  
Wall Thickening ◽  
Papilla Of Vater ◽  
Extrahepatic Bile Duct Cancer ◽  
Bile Duct Stenosis ◽  
Cancer Spread ◽  
Duct Stenosis ◽  
Maximum Minimum

The characteristics of sites of intramural cancer spread were examined by comparing the intraductal ultrasonography (IDUS) and wall thickening findings at sites of intramural cancer spread and non-spread, in patients with malignant bile duct stenosis who had undergone percutaneous transhepatic biliary drainage (PTBD).The subjects were ten patients with extrahepatic bile duct cancer, two with pancreatic cancer, one with cancer of the gallbladder, and one with cancer of the papilla of Vater who underwent preoperative IDUS. From these patients, 50 IDUS slices were examined with a congruent relationship with the histologic section of resected tissue. The maximum thickening, minimum thickening, maximum/minimum thickening ratio, and form factor of the medial and lateral margins of the medial hypoechoic layer were determined using diagnostic imaging, and the results were compared at sites of cancer spread and non-spread.Twelve slices were obtained from the site of stenosis, 14 from sites of cancer spread, and 24 from non-spread sites. The maximum thickening, minimum thickening, and maximum/minimum thickening ratio differed significantly between the sites of spread and the non-spread.The absolute values for wall thickening are useful for diagnosing the presence of intramural spread in patients with malignant biliary duct stenosis.

Simultaneous occurrence of autoimmune pancreatitis and sclerosing cholangitis as immune-related adverse events of pembrolizumab

2021 ◽  
Vol 14 (6) ◽  
pp. e243360
Author(s):  
Tsuyoshi Suda ◽  
Masako Kobayashi ◽  
Koji Kurokawa ◽  
Eiki Matsushita
Keyword(s):  
Bile Duct ◽  
Adverse Events ◽  
Autoimmune Pancreatitis ◽  
Sclerosing Cholangitis ◽  
Intrahepatic Bile Duct ◽  
Simultaneous Occurrence ◽  
Cd8 Cells ◽  
Igg4 Related Disease ◽  
Contrast Enhanced Ct ◽  
Serum Igg4

A 57-year-old man with lung cancer, previously treated with the programmed death-1 inhibitor pembrolizumab, was evaluated for liver injury and acute pancreatitis. Serum IgG4 levels were not elevated. Contrast-enhanced CT showed pancreatic swelling, contrast unevenness in the liver and thickening of the common bile duct and gall bladder. Magnetic resonance cholangial pancreatography revealed beads in the left intrahepatic bile duct and localised narrowing of the head and body of the central pancreatic duct. Endoscopic ultrasound-guided fine-needle and liver needle biopsy showed CD8+ and CD4+ T lymphocyte aggregates, whereas immunostaining revealed greater infiltration by CD8+ cells than CD4+ cells. IgG4-related disease was ruled out based on serum and pathological findings. The patient simultaneously presented with immune-related adverse events, autoimmune pancreatitis-like features and sclerosing cholangitis, which were ameliorated by steroid therapy. CD8+ lymphocytes were the dominant infiltrating cells in autoimmune pancreatitis and sclerosing cholangitis.

scholarly journals Role of Peroral Cholangioscopy in the Diagnosis of Primary Sclerosing Cholangitis

Diagnostics ◽  
2020 ◽  
Vol 10 (5) ◽  
pp. 268
Author(s):  
Toshio Fujisawa ◽  
Mako Ushio ◽  
Sho Takahashi ◽  
Wataru Yamagata ◽  
Yusuke Takasaki ◽  
...  
Keyword(s):  
Bile Duct ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Chronic Phase ◽  
Active Phase ◽  
Future Studies ◽  
Bile Duct Stenosis ◽  
Peroral Cholangioscopy ◽  
Duct Stenosis

Primary sclerosing cholangitis (PSC) is characterized by idiopathic biliary stricture followed by progressive cholestasis and fibrosis. When diagnosing PSC, its differentiation from other types of sclerosing cholangitis and cholangiocarcinoma is necessary. The cholangioscopic findings of PSC have not been investigated sufficiently. PSC and IgG4-related sclerosing cholangitis are difficult to distinguish by peroral cholangioscopy (POCS), but POCS is useful for excluding cholangiocarcinoma. POCS findings vary according to the condition and stage of disease. In the active phase, findings such as mucosal erythema, ulceration, fibrinous white exudate, and an irregular surface are observed and may reflect strong inflammation in the biliary epithelium. On the other hand, findings such as scarring, pseudodiverticula, and bile duct stenosis appear in the chronic phase and may reflect fibrosis and stenosis resulting from repeated inflammation. Observation of inside the bile duct by POCS might confirm the current PSC activity. Because POCS offers not only information regarding the diagnosis of PSC and PSC-associated cholangiocarcinoma but also the current statuses of biliary inflammation and stenosis, POCS could significantly contribute to the diagnosis and treatment of PSC once the characteristic findings of PSC are confirmed by future studies.

scholarly journals IgG4-Related Sclerosing Cholangitis Involving the Intrahepatic Bile Ducts Diagnosed with Liver Biopsy

2018 ◽  
Vol 2018 ◽  
pp. 1-7
Author(s):  
Malene Theilmann Thinesen ◽  
Ove B. Schaffalitzky de Muckadell ◽  
Sönke Detlefsen
Keyword(s):  
Bile Duct ◽  
Liver Biopsy ◽  
Hot Spots ◽  
Sclerosing Cholangitis ◽  
Bile Ducts ◽  
Bile Duct Carcinoma ◽  
Duct Carcinoma ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4

IgG4-related disease is characterized by lymphoplasmacytic inflammation and fibrosis, often leading to mass-forming lesions in different organs. When IgG4-related disease affects the bile ducts, it is called IgG4-related sclerosing cholangitis. A 74-year-old male complained of dysphagia and abdominal pain. Endoscopic retrograde cholangiography and magnetic resonance cholangiography revealed bile duct changes suspicious of a bile duct carcinoma or cholangitis. Liver biopsy showed storiform fibrosis, lymphoplasmacytic infiltration, obliterative phlebitis, and a portal-based inflammatory nodule with expansion of a portal tract. Hot spots revealed 339 IgG4-positive cells per high power field (HPF) and an IgG4/IgG ratio of 72%. Eight months earlier, an inguinal lymph node had been removed, showing expanded interfollicular zones and increased plasma cells. Hot spots revealed 593 IgG4-positive cells and an IgG4/IgG ratio of 92%. The serum IgG4 of the patient was elevated nearly 10 times upper limit of normal. The diagnosis of IgG4-related sclerosing cholangitis associated with IgG4-related lymphadenopathy was made. There was good response to treatment with prednisolone and azathioprine. The differentiation of IgG4-related sclerosing cholangitis from primary sclerosing cholangitis and bile duct carcinoma is often difficult. Liver biopsy only rarely contributes to this setting, but we describe and report in detail a case where liver biopsy showed a portal-based inflammatory nodule with the characteristic features of this disease.

Sclerosing Cholangitis Related to IgG4: Not Always a Curable Entity

2018 ◽  
Vol 17 (5) ◽  
pp. 0-10
Author(s):  
Fernando Martínez-Valle ◽  
Mar Riveiro-Barciela ◽  
María Teresa-Salcedo ◽  
Xavier Merino-Casabiel ◽  
Andreu Fernández-Codina ◽  
...  
Keyword(s):  
Sclerosing Cholangitis ◽  
Good Prognosis ◽  
Biliary Cirrhosis ◽  
Histopathological Findings ◽  
Systemic Involvement ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Early Use ◽  
Secondary Biliary Cirrhosis

IgG4-related disease is a recently-described fibro-inflammatory condition with characteristic histopathological findings in the organs involved. The most commonly affected organs are pancreas, lymph nodes, and retroperitoneum. Liver disease usually involves bile structures and therefore IgG4-related disease is considered a cause of secondary sclerosing cholangitis. One out of three patients with IgG4 sclerosing cholangitis also presents autoimmune pancreatitis, although it can be associated with manifestations in other organs. One of the main features of IgG4-related disease is its good prognosis due to the great response to glucocorticoid therapy. However, relapse of the disease is not uncommon, especially when steroid therapy is decreased or stopped. Rituximab seems to be an effective treatment to achieve remission of the disease. We report the case of a 74 year-old man diagnosed with IgG4-related disease based on increase of serum IgG4 levels, imaging and histopathological findings, with systemic involvement including sclerosing cholangitis. Despite the absence of liver fibrosis at onset, the early use of glucocorticoids and rituximab therapy, the patient presented clinical and analytical deterioration, leading to secondary biliary cirrhosis. In conclusion, this clinical case highlights the importance

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